Gastroschizis Vs Omfalocel
Gastroschizis Vs Omfalocel
Gastroschizis Vs Omfalocel
Omphalocele
Dr. Kevin Boykin Pediatric Surgery
Take Home #1
Prior to the late 1960s, survival for infants with gastroschisis was rare. Three major advances have led to marked improvement:
Parenteral nutrition Silo closure Advances in perinatal (NICU) care
Embryology
Embryo unfolds in four different directions:
Cranial (thoracic & epigastric walls) Caudal (hindgut, bladder, hypogastric wall) Lateral (midgut and lateral abdominal wall)
Midgut grows rapidly from 6-10 weeks Midgut elongates faster than the abdominal wall and herniates through the umbilical ring
Embryology
Normal development:
Midgut rotates and returns into abdominal cavity by week 10-12 Allantois, vitelline duct, vessels obliterate Umbilicus with vein and two arteries remains Umbilical ring closes around these structures
Pathogenesis
Poorly understood Depends on timing of embryologic abnormality:
The earlier the event, the more complex the anomaly
7-8 Weeks
Pentalogy of Cantrell
Thoracoabdominal ectopia cordis Cardiac defects Omphalocele Cleft sternum Diaphragmatic, pericardial defects
7-8 Weeks
Cloacal Exstrophy
Extrophy of the bladder Imperforate anus Omphalocele Lower neural tube defects
7-8 Weeks
Omphalocele
Improper migration of fusion of the lateral folds resulting in failure of closure of the umbilical ring = persistent herniation of midgut Umbilical vessels insert on to the sac and radiate around it (important remember this)
Etiology of Defects
Younger mothers <20 Folic acid deficiency Hypoxia Salicylates, acetaminophen, ibuprofen, pseudoephedrine Marijuana, cocaine, alcohol
Genetics
Gastroschisis
Hirschsprungs disease Oromandibular limb hypogenesis (sporadic)
Omphalocele
Trisomy 13 Trisomy 14 Trisomy 15 Trisomy 18 Trisomy 21
Incidence
Gastroschisis
1 in 6,000 Male = female Incidence increasing
Omphalocele
1 in 4,000 Male = female Incidence is static
Take Home #2
The physiologic impact of omphalocele is related to its size and associated anomalies:
GI FUNCTION IS NORMAL!
In gastroschisis the bowel does not function properly but the child rarely has other serious issues.
Umbilical cord Left of defect Bowel Alimentation Anomalies Inflamed Delayed 10%
Gastroschisis
Intestinal atresia Small for gestational age Prematurity GERD Cryptorchidism
Gastroschisis
Small defect (usually less than 4 cm) Occurs to the right of the umbilicus Normal abdominal wall and musculature No sac or remnant of a sac! Midgut herniated through defect
Possibly duodenum, stomach, colon or gonad
Associated Conditions
Intestinal atresia 10% GERD 16% Undescended testicle 15%
Usually corrects spontaneously
Gastroschisis Pearls
Close primarily if possible Silo reduction is good alternative Dont make the closure too tight Wait to feed the patient (14 days) Slow advances on feeds Expect high residuals and bilious aspirates Rectal stimulation
Postop Problems
Necrotizing Enterocolitis Short gut syndrome Malabsorption Obstruction TPN-associated cholestasis Developmental delay
Characterized by: prolonged ileus & time to tolerate feeds, longer length of stay, increased infectious complication & mortality.
Omphalocele
Omphalocele
At birth look for other anomalies
Echocardiogram CXR Renal ultrasound
Other more severe associated anomalies take precedence over surgical repair
Exception: disruption of the sac
Omphalocele
If severe pulmonary hypoplasia is present:
Closure of abdomen may be impossible Treat with topical bacteriocidal agent
Wait for epithelialization Fix hernia at later date
Small Omphalocele
Usually closed primarily Very similar to gastroschisis closure Watch abdominal pressure Ligate umbilical vessels Neoumbilicus can be created
Large/Giant Omphalocele
Options include:
Short-term silo reduction (2 weeks)
Followed by closure with mesh
Postop Management
Respiratory support is critical
Intubation with paralysis
Nutritional support
TPN initially
REMEMBER
Omphalocele has normal GI function but bad associated anomalies/defects survival about 60% Gastroschisis has horrible GI function but few associated anomalies/defects survival about 85-90%